Young William F
Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic; and Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Clin Endocrinol (Oxf). 2007 May;66(5):607-18. doi: 10.1111/j.1365-2265.2007.02775.x.
Great strides have been made in our understanding of the pathophysiology of primary aldosteronism syndrome since Conn's description of the clinical presentation of a patient with an aldosterone-producing adenoma (APA) more than 50 years ago. It is now recognized that the APA is just one of the seven subtypes of primary aldosteronism. APA and bilateral idiopathic hyperaldosteronism (IHA) are the most common subtypes of primary aldosteronism. Although most clinicians had thought primary aldosteronism to be a rare form of hypertension for more than three decades, it is now recognized to be the most common form of secondary hypertension. Using the plasma aldosterone to plasma renin activity ratio as a case-finding test, followed by aldosterone suppression confirmatory testing, has resulted in much higher prevalence estimates of 5-13% of all patients with hypertension. In addition, there has been a new recognition of the aldosterone-specific cardiovascular morbidity and mortality associated with aldosterone excess. Although thought to be daunting and complex in the past, the diagnostic approach to primary aldosteronism is straightforward and can be considered in three phases: case-finding tests, confirmatory tests and subtype evaluation tests. Patients with hypertension and hypokalaemia (regardless of presumed cause), treatment-resistant hypertension (three antihypertensive drugs and poor control), severe hypertension (>or= 160 mmHg systolic or >or= 100 mmHg diastolic), hypertension and an incidental adrenal mass, onset of hypertension at a young age or patients being evaluated for other forms of secondary hypertension should undergo screening for primary aldosteronism. In patients with suspected primary aldosteronism, screening can be accomplished by measuring a morning (preferably between 0800 and 1000 h) ambulatory paired random plasma aldosterone concentration (PAC) and plasma renin activity (PRA). An increased PAC:PRA ratio is not diagnostic by itself, and primary aldosteronism must be confirmed by demonstrating inappropriate aldosterone secretion. Aldosterone suppression testing can be performed with orally administered sodium chloride and measurement of urinary aldosterone or with intravenous sodium chloride loading and measurement of PAC. Unilateral adrenalectomy in patients with APA or unilateral adrenal hyperplasia results in normalization of hypokalaemia in all these patients; hypertension is improved in all and is cured in approximately 30-60% of them. In bilateral adrenal forms of primary aldosteronism, unilateral or bilateral adrenalectomy seldom corrects the hypertension and they should be treated medically with a mineralocorticoid receptor antagonist.
自从50多年前康恩描述了一例醛固酮分泌腺瘤（APA）患者的临床表现以来，我们对原发性醛固酮增多症综合征病理生理学的理解有了长足的进步。现在人们认识到，APA只是原发性醛固酮增多症的七种亚型之一。APA和双侧特发性醛固酮增多症（IHA）是原发性醛固酮增多症最常见的亚型。尽管在三十多年的时间里，大多数临床医生都认为原发性醛固酮增多症是一种罕见的高血压形式，但现在它被认为是继发性高血压最常见的形式。使用血浆醛固酮与血浆肾素活性比值作为筛查试验，随后进行醛固酮抑制确诊试验，已使所有高血压患者中原发性醛固酮增多症的患病率估计值大幅提高至5% - 13%。此外，人们对与醛固酮过量相关的特定心血管发病率和死亡率有了新的认识。尽管过去认为原发性醛固酮增多症的诊断方法令人生畏且复杂，但它其实很直接，可分为三个阶段：筛查试验、确诊试验和亚型评估试验。患有高血压和低钾血症（无论推测病因如何）、难治性高血压（使用三种抗高血压药物且控制不佳）、重度高血压（收缩压≥160 mmHg或舒张压≥100 mmHg）、高血压且伴有肾上腺意外瘤、年轻时发病的高血压或正在接受其他形式继发性高血压评估的患者，都应接受原发性醛固酮增多症的筛查。对于疑似原发性醛固酮增多症的患者，筛查可通过测量早晨（最好在08:00至10:00之间）动态配对随机血浆醛固酮浓度（PAC）和血浆肾素活性（PRA）来完成。PAC:PRA比值升高本身并不能确诊，必须通过证明醛固酮分泌不适当来确诊原发性醛固酮增多症。醛固酮抑制试验可通过口服氯化钠并测量尿醛固酮，或通过静脉输注氯化钠负荷并测量PAC来进行。对于APA或单侧肾上腺增生患者，单侧肾上腺切除术可使所有这些患者的低钾血症恢复正常；所有患者的高血压都有所改善，约30% - 60%的患者可治愈。在原发性醛固酮增多症的双侧肾上腺形式中，单侧或双侧肾上腺切除术很少能纠正高血压，应使用盐皮质激素受体拮抗剂进行药物治疗。
